8.3 Mesotympanic Cholesteatoma
8.4 Cholesteatoma Associated with Atelectasis
8.5 Cholesteatoma Associated with Complications
8.6 Surgical Treatment of Cholesteatoma: Individualized Technique
8 Chronic Suppurative Otitis Media with Cholesteatoma
Keywords: epitympanic cholesteatoma, mesotympanic cholesteatoma, closed tympanoplasty, open tympanoplasty, modified Bondy’s technique
Abstract
Cholesteatoma is an epidermal inclusion cyst localized in the middle ear. The “acquired type” can be caused by invasion of the skin of the external auditory canal into the middle ear through a marginal perforation. It can also originate from an epitympanic retraction pocket that becomes so deep that keratin debris can no longer be expelled, leading to its accumulation and subsequent cholesteatoma formation. Such retraction pockets can remain asymptomatic until they become infected, resulting in otorrhea and hearing loss. For this reason, we prefer to follow up these patients with otomicroscopy and endoscopy. In rare cases, the cholesteatoma can invade the labyrinth, cochlea, posterior and middle fossa durae, the internal auditory canal, and the petrous apex, forming a petrous bone cholesteatoma. Treatment of cholesteatoma is exclusively surgical and individualized (tympanoplasty).
Cholesteatoma is an epidermal inclusion cyst localized in the middle ear, whose capsule and matrix are formed from stratified squamous epithelium. The desquamating debris includes pearly white lamellae of keratin that accumulate concentrically, forming the cholesteatoma mass.
The term cholesteatoma is actually a misnomer. It is derived from the Greek words “chole” or bile, “steatos” or fat, and “oma” or tumor. There is no relation between cholesteatoma and bile or fat. The suffix “oma” (tumor), however, is more appropriate because cholesteatoma can be considered an epidermal inclusion cyst.
Cholesteatoma can be divided into congenital (middle ear or petrous bone) and acquired (middle ear or petrous bone). Congenital cholesteatoma arises as a result of entrapped ectodermal cellular debris during embryonic development. When it involves the middle ear, it appears as a whitish retrotympanic mass that may be localized either anterior or posterior to the malleus (see Chapter ▶ 9). When it involves the petrous part of the temporal bone, it is termed congenital petrous bone cholesteatoma and in the majority of cases it is localized in the petrous apex (see Chapter ▶ 10). In this chapter, we will deal exclusively with cholesteatoma involving the middle ear. Petrous bone cholesteatoma is dealt with in a later chapter.
Acquired cholesteatoma of the middle ear can be caused by invasion of the skin of the external auditory canal into the middle ear through a marginal perforation. It can also originate from an epitympanic retraction pocket that becomes so deep that keratin debris can no longer be expelled, leading to its accumulation and subsequent cholesteatoma formation. Such retraction pockets can remain asymptomatic until they become infected, resulting in otorrhea and hearing loss. In other cases, the only symptom might be progressive hearing loss due to erosion of the ossicular chain by the developing cholesteatoma.
Because it is not always easy to establish a clear distinction between epitympanic or posterosuperior retraction pockets and cholesteatoma, we prefer to follow up these patients with otomicroscopy and endoscopy. In cases in which the retraction pocket becomes deep, giving rise to a cholesteatoma, a tympanoplasty is indicated. Because of the early stage of the disease, surgery can be done in a single stage. Fetid otorrhea and hearing loss are the main complaints in cholesteatoma. In addition, complicated cases can manifest with vertigo and/or facial nerve paralysis. Vertigo occurs as a result of labyrinthine fistula, which is most commonly located in the lateral semicircular canal. Facial paralysis can be caused by pressure of the cholesteatoma sac or neuritis.
In rare cases, the cholesteatoma can invade the labyrinth, cochlea, posterior and middle fossa durae, the internal auditory canal, and the petrous apex, forming a petrous bone cholesteatoma (see Chapter ▶ 10).
Treatment of cholesteatoma is exclusively surgical. Early in this century, radical mastoidectomy, a destructive procedure for the middle ear, was performed with the only goal being eradication of infection to save the ear and the life.
The concept of tympanoplasty was introduced in early 1950s. Tympanoplasty was aimed at eradication of infection as well as reconstruction of the tympano-ossicular system. Today, two types of tympanoplasty are employed: closed tympanoplasty, in which the posterior canal wall is preserved, and open tympanoplasty, in which the posterior canal wall is drilled. Both techniques, when performed appropriately and with the proper indications, can produce excellent results in terms of eradication of cholesteatoma and restoration of hearing. In children, the closed technique, performed in two stages, is preferred in the majority of cases due to children’s highly cellular mastoids and in an attempt to preserve the anatomy of the ear as much as possible. In adults, particularly in epitympanic cholesteatoma with marked erosion of the scutum, in cases with sclerotic mastoids, or when middle ear atelectasis is present, an open tympanoplasty is performed (see also Chapter ▶ 14).
8.1 Epitympanic Retraction Pocket
See ▶ Fig. 8.1, ▶ Fig. 8.2, ▶ Fig. 8.3, ▶ Fig. 8.4, ▶ Fig. 8.5, ▶ Fig. 8.6, ▶ Fig. 8.7.
Fig. 8.1 Right ear. Early epitympanic retraction pocket. The tympanic membrane shows grade I atelectasis. Middle ear effusion with characteristic yellowish coloration of the drum is seen. In the anterosuperior quadrant, the tubal orifice is visible in transparency, whereas the long process of the incus is evident in the posterosuperior quadrant. In the area of the cone of light, an atrophic part of the tympanic membrane due to a precious myringotomy can be appreciated.
Fig. 8.2 Right ear. Epitympanic retraction pocket with the onset of tympanosclerosis of the pars tensa of the tympanic membrane.
Fig. 8.3 Right ear. Similar case. The anterior quadrants of the pars tensa are retracted and thickened.
Fig. 8.4 Right ear. Epitympanic pocket. The neck of the malleus is visible.
Fig. 8.5 Right ear. A large controllable epitympanic retraction pocket with erosion of the scutum. The head of the malleus is seen. Middle ear effusion gives the tympanic membrane the characteristic yellowish coloration. To prevent progression of the retraction pocket and formation of adhesions, myringotomy, ventilation tube insertion, and regular follow-up are indicated. These cases frequently represent the transition from a simple retraction pocket to an initial attic cholesteatoma. The distinction between the two is sometimes difficult. In suspected cases, a high-resolution computed tomography (CT) scan (with bone window) is beneficial for better evaluation of the extension of the retraction pocket. In cases where the condition remains stable with regular follow-up and where hearing is normal, no surgery is required. If the pocket extends deeper, giving rise to a frank cholesteatoma, surgery is indicated. If hearing is normal, an open tympanoplasty (modified Bondy’s technique) is performed in a single stage.
Fig. 8.6 Right ear. In this case, the retraction pocket is deeper than in ▶ Fig. 8.4. Even if the CT scan did not show a frank cholesteatoma, the patient referred occasional otorrhea. So, he underwent surgery (closed tympanoplasty with attic reconstruction with cartilage and bone pate). No cholesteatoma was found in the cavity.
Fig. 8.7 Left ear. Deep epitympanic erosion extended to the posterior epitympanum. The neck of the malleus and the body of the incus are under view. The chorda tympani is also evident. The patient did not complained of otorrhea or hearing loss, but a strict follow-up should be assessed to avoid further development of a cholesteatoma.
8.2 Epitympanic Cholesteatoma
See ▶ Fig. 8.8, ▶ Fig. 8.9, ▶ Fig. 8.10, ▶ Fig. 8.11, ▶ Fig. 8.12, ▶ Fig. 8.13, ▶ Fig. 8.14, ▶ Fig. 8.15, ▶ Fig. 8.16, ▶ Fig. 8.17, ▶ Fig. 8.18, ▶ Fig. 8.19, ▶ Fig. 8.20, ▶ Fig. 8.21, ▶ Fig. 8.22, ▶ Fig. 8.23, ▶ Fig. 8.24, ▶ Fig. 8.25, ▶ Fig. 8.26, ▶ Fig. 8.27, ▶ Fig. 8.28, ▶ Fig. 8.29, ▶ Fig. 8.30, ▶ Fig. 8.31, ▶ Fig. 8.32, ▶ Fig. 8.33, ▶ Fig. 8.34, ▶ Fig. 8.35, ▶ Fig. 8.36, ▶ Fig. 8.37, ▶ Fig. 8.38.
Fig. 8.8 Left ear. Epitympanic cholesteatoma in a 20-year-old man in the only hearing ear. The whitish cholesteatoma mass is visible anterior to the malleus (arrow). This is an example of how an epitympanic pocket can progressively led to a cholesteatoma. This patient underwent removal of a right petrous bone massive cholesteatoma (for definition and classification, see Chapter ▶ 10) through a transotic approach 5 years before. The left epitympanic retraction pocket (see ▶ Fig. 8.9) was followed up until the evidence of a cholesteatoma. The patient did not complain of any symptom on the left ear, and hearing function was normal. Considering the small size of the cholesteatoma, a left closed tympanoplasty was performed with preservation of the whole ossicular chain (see ▶ Fig. 8.12, ▶ Fig. 8.13, ▶ Fig. 8.14, ▶ Fig. 8.15, ▶ Fig. 8.16). A detailed description of the steps of open and closed tympanoplasties will be provided at the end of the chapter.
Fig. 8.9 Same case. Otoscopy performed 1 year before that on ▶ Fig. 8.8. A small controllable anteromalleolar epitympanic retraction pocket is visible (arrow). Considering that it is the only hearing ear, surgery is questionable at this stage, but a strictly follow-up is mandatory because early detection of a cholesteatoma is crucial for a correct management and outcome.
Fig. 8.10 CT scan, coronal view, of the same case. A small epitympanic cholesteatoma is visible (arrow).
Fig. 8.11 CT scan, axial view, of the same case.
Fig. 8.12 Intraoperative picture of the same case. Canal wall-up mastoidectomy has been performed. The short process of the incus is visible. The mastoid is free from pathology. I, incus; MFD, middle fossa dura; PCW, posterior canal wall; SS, sigmoid sinus.
Fig. 8.13 The tympanic membrane is reflected anteroinferiorly and the cholesteatoma is visible anteromedially to the handle of the malleus. Ch, cholesteatoma; M, malleus; TM, tympanic membrane.
Fig. 8.14 The cholesteatoma is progressively removed with the help of the suction and Cottonoids (see ▶ Fig. 8.15).
Fig. 8.15 Cottonoids are used to clean the medial surface of the handle of the malleus to avoid any residual pathology. In cases like this (cholesteatoma in the only hearing ear), it is of utmost importance to be radical in disease extirpation and careful to preserve structure and function of the middle ear.
Fig. 8.16 View after disease clearance. The malleus and the middle ear cleft are free from the cholesteatoma. A 0- or 30-degree endoscope could be used to further check for any residual pathology. At the end of the procedure, the tympanic membrane is reflected and reconstructed/reinforced with a piece of fascia of the temporalis muscle, while the middle ear is filled with Gelfoam (see Chapter ▶ 7 for myringoplasty).
Fig. 8.17 Left ear. Epitympanic erosion with cholesteatoma. The head of the malleus and the supratubal recess are visible.
Fig. 8.18 Left ear. Epitympanic cholesteatoma. The head of the malleus and the body of the incus are visible. The inferior and posterior quadrants of the tympanic membrane are tympanosclerotic. The cholesteatoma was lateral to the ossicular chain (see ▶ Fig. 8.19). Hearing function was normal. A modified Bondy’s technique tympanoplasty, was performed and no recurrence of cholesteatoma occurred during the follow-up.
Fig. 8.19 CT of the previous case, coronal view. The cholesteatoma is located in the epitympanic area (arrow), lateral to the malleus. The middle ear is free.
Fig. 8.20 Large epitympanic erosion with cholesteatoma. The head of the malleus and the body of the incus are eroded.
Fig. 8.21 Epitympanic cholesteatoma with erosion of the head of the malleus and the incus. The mass is in contact with the stapes (arrow), resulting in normal hearing. The rest of the tympanic membrane is slightly retracted (with myringostapedopexy) due to obstruction of the Eustachian tube. In cases like this, surgery could probably worsen the hearing function (with resulting conductive hearing loss), so the patient has to be informed for an eventual second-stage ossiculoplasty.
Fig. 8.22 Same patient as in ▶ Fig. 4.6, contralateral side. Large epitympanic erosion with cholesteatoma. Erosion of the head of the malleus is also visible. The CT scan (see ▶ Fig. 8.23, ▶ Fig. 8.24, ▶ Fig. 8.25) also showed involvement by the pathology at the level of the incudostapedial joint, antrum, and mastoid. Audiometry showed moderate conductive hearing loss. In this case, an open tympanoplasty is the treatment of choice. A second stage with ossiculoplasty could be eventually performed in case of poor hearing recovery or to exclude residual disease, after 10 to 12 months, via a transcanal approach.
Fig. 8.23 CT scan, coronal view. Epitympanic cholesteatoma with erosion of the head of the malleus (red arrow) and thinning of the tegmen tympani (green arrow).
Fig. 8.24 CT scan, coronal view. The long process of the incus and stapes suprastructure seem involved and eroded by the cholesteatoma.
Fig. 8.25 CT scan, axial view. The cholesteatoma extends posteriorly toward the antrum and the mastoid (arrow).
Fig. 8.26 Another case of epitympanic cholesteatoma. There is a deep erosion with involvement of the malleus and the incus.
Fig. 8.27 CT scan, coronal view, of the same case. Epitympanic cholesteatoma with erosion of the long process of the incus (arrow).
Fig. 8.28 Right ear. Epitympanic erosion with cholesteatoma. The tympanic membrane is completely tympanosclerotic. The patient did not complain of otorrhea (dry cholesteatoma).
Fig. 8.29 Right ear of a 46-year-old patient suffering from bilateral cholesteatoma. An epitympanic erosion with cholesteatoma and middle ear effusion showing an air–fluid level can be seen. CT scan ( ▶ Fig. 8.31) demonstrates cholesteatoma extension into the mastoid. Intraoperatively, a fistula of the lateral semicircular canal was encountered, as well as erosion of the incus. A single-stage open tympanoplasty was performed with autologous incus interposition between the handle of the malleus and the head of the stapes. In patient with bilateral cholesteatoma, an open technique is preferred.
Fig. 8.30 Left ear of the same patient. Cholesteatoma with marked erosion of the scutum and epidermization of the attic and mesotympanum. The cholesteatoma debris was partially cleaned. The residual pars tensa shows tympanosclerosis. Intraoperatively, the ossicular chain was absent. The otoscopic view of the left ear is apparently more advanced than the right ear. This, however, was not the case intraoperatively since the marked epitympanic erosion shown here allowed self-cleaning of the cholesteatoma debris (see CT scan, ▶ Fig. 8.31). Because of the total destruction of the ossicular chain, a second stage was programmed for functional reconstruction.
Fig. 8.31 CT of the previous case showing cholesteatoma extension in the mastoid in the right ear and self-cleaning of the cholesteatoma debris in the left ear (arrows).
Fig. 8.32 Right ear. Large epitympanic erosion with cholesteatoma. This 18-year-old patient did not have otorrhea. Ipsilateral hearing was normal, whereas the contralateral side showed severe sensorineural hearing loss secondary to previous surgery of radical mastoidectomy. Given the intact ossicular chain, an open tympanoplasty (modified Bondy’s technique) was performed. According to our strategy, cholesteatoma in the only hearing ear is one of the absolute indications for performing an open technique. The reason is that this technique, if properly performed, ensures complete eradication of the pathology and better long-term follow-up, thus minimizing the risk of recurrence. Further surgical interventions, with their potential risk even in the most experienced hand, are therefore avoided.
Fig. 8.33 Right ear. Large epitympanic erosion with cholesteatoma and polypoid tissue that covers the head of the malleus. The pars tensa is intact.
Fig. 8.34 Left ear. Epitympanic cholesteatoma. Extensive erosion of the scutum with excessive cholesteatomatous debris. The pars tensa shows grade I atelectasis with catarrhal middle ear effusion.
Fig. 8.35 Left ear. Cystic retrotympanic cholesteatoma situated posterior to the malleus. The tympanic membrane shows bulging at the level of the pars flaccida and slight retraction with tympanosclerosis in the posterior quadrants.
Fig. 8.36 Same case as in ▶ Fig. 8.35 during an acute inflammatory episode. Note the increase in size of the cholesteatomatous cyst.
Fig. 8.37 Left ear. A large epitympanic erosion is seen with epidermization of the attic and posterior mesotympanum. The cholesteatoma, visible in transparency, causes bulging of the tympanic membrane in the posterior inferior quadrants. Resorption of the incus and head of the malleus is discernible.
Fig. 8.38 Right ear. Epitympanic erosion with cholesteatoma. Extension of the cholesteatoma into the mesotympanum is seen through the bulging posterior quadrants of the tympanic membrane.
Summary
An epitympanic retraction pocket should be regularly checked with otomicroscopy. The 30-degree rigid endoscope allows visualization of the extent of the retraction pocket that can be difficult with the microscope. When progression of the epithelium into the epitympanum cannot be controlled, the presence of cholesteatoma is considered. In such cases, surgery should be performed. Whenever a minor epitympanic erosion is present, we adopt a closed technique with reconstruction of the attic using a cartilage and bone paste. This technique is valid particularly in children, in whom the mastoid is usually very pneumatized. Frequently, surgery is staged in these cases.
When a marked attic erosion is present, especially in adults, we perform an open technique to avoid cholesteatoma recurrence that can occur due to absorption of the material used for reconstruction of the attic defect. When preoperative hearing is normal in the presence of attic cholesteatoma with large bony erosion, we perform an open tympanoplasty in the form of a modified Bondy’s technique. This technique allows single-stage eradication of the disease with conservation of the normal preoperative hearing.
Fig. 8.39 Right ear. Mesotympanic cholesteatoma. The epithelial squamae can be seen through the retromalleolar perforation. Anterior to the malleus, the cholesteatoma mass causes bulging and whitish coloration of the tympanic membrane without perforating it. The entire middle ear is filled with cholesteatoma in this case.
8.3 Mesotympanic Cholesteatoma
See ▶ Fig. 8.39, ▶ Fig. 8.40, ▶ Fig. 8.41, ▶ Fig. 8.42, ▶ Fig. 8.43, ▶ Fig. 8.44, ▶ Fig. 8.45, ▶ Fig. 8.46, ▶ Fig. 8.47, ▶ Fig. 8.48, ▶ Fig. 8.49, ▶ Fig. 8.50, ▶ Fig. 8.51, ▶ Fig. 8.52, ▶ Fig. 8.53, ▶ Fig. 8.54, ▶ Fig. 8.55, ▶ Fig. 8.56.
Fig. 8.40 Right ear. Posterior mesotympanic cholesteatoma associated with a polyp are seen at the level of the oval window. There is evidence of discharge.
Fig. 8.41 Left ear. Small epitympanic erosion and mesotympanic retraction pocket with wax and cholesteatomatous squamae. Extension of the cholesteatomatous mass into the anteromalleolar region is seen through the retracted tympanic membrane.
Fig. 8.42 Right ear. Posterior perforation with cholesteatoma in the posterior mesotympanum. The cholesteatomatous squamae cover the region of the oval window extending toward the attic and progress anterior to and under the handle of the malleus. The promontory and the round window are visible through the perforation.
Fig. 8.43 Right ear. Total tympanic membrane perforation. The handle of the malleus is absent. The long process of the incus and part of the stapes are covered by cholesteatoma, which also involves the promontory. The round window, hypotympanic air cells, and tubal orifice are free from the pathology. In these cases, a staged closed tympanoplasty can be performed.
Fig. 8.44 Mesotympanic cholesteatoma. The long process of the incus is surrounded by cholesteatoma. The rest of the tympanic membrane is tympanosclerotic. In this case, a staged open tympanoplasty is the treatment of choice.
Fig. 8.45 Mesotympanic retraction pocket with cholesteatoma. An epitympanic retraction is also visible. The incudostapedial joint is completely epithelialized (arrow).
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